Gastrointestinal Manifestations of Crohn’s Disease
The gastrointestinal manifestations of Crohn’s disease (CD) and ulcerative colitis (UC) often overlap. Increased stool frequency with decreased stool consistency are the most common presenting symptoms associated with CD and UC, although alterations in bowel habits are not always present, and patients with distal disease may present with constipation.2034 Diarrhea is present in the majority of patients with CD. Cramping pain prior to bowel movements that is relieved after defecation is commonly associated with UC. Distal inflammation with UC is often accompanied by tenesmus, a constant feeling of the need to have a bowel movement. Patients may complain of a sense of urgency, leading to fear of incontinence. The presence of blood in the stool is reported by almost all patients with UC.2112 Pain is also common in CD, with patient reports of generalized abdominal pain or cramping in the right lower quadrant. The presence of bright red blood and tenesmus are more frequently associated with UC, due to the distal nature of the disease. In contrast, patients with CD may experience "rectal sparing," in that the rectum is relatively free from inflammation. Blood may or may not be visible in the stool of patients with CD, but stool tests for occult blood are often positive during active disease. Weight loss may be seen in both forms of inflammatory bowel disease (IBD) but is more common in CD due to involvement of the absorptive surface of the small bowel, metabolic demands of the disease, and decreased dietary intake. According to published literature, children with IBD may demonstrate prominent extraintestinal manifestations, including growth failure and delayed puberty.11432
In one study, investigators evaluated data collected in a pediatric IBD registry to examine disease characteristics on presentation, and to compare disease course by age of onset in children diagnosed before the age of 18. Among 940 children with CD, 83 were diagnosed between the ages of 0 and 5 years, while 857 were between 6 and 17 years of age. In the younger cohort, there was no difference related to severity of disease at onset; however, children in the older age group were more likely to present with fever, weight loss, and abdominal pain, while the younger children were more likely to present with rectal bleeding. There was a significantly higher rate of some disease-related complications (abscess, fistulas, stricture, fissure) observed in the older age group. Other complications, such as osteoporosis, growth failure, autoimmune hepatitis, aphthous stomatitis, and clubbing did not differ by age group.11433
There are few studies that have been conducted to examine the features of CD in the pediatric population. In a population-based study, Kugathasan and colleagues described epidemiologic and clinical characteristics of IBD in newly diagnosed children in Wisconsin during a 2-year period from January 1, 2000 through December 31, 2001. In this study, 199 incident cases of IBD were identified through endoscopy and mucosal biopsies by 21 pediatric gastroenterologists who participated. Of these 199 cases, the majority of cases (64.8%) were pediatric CD. The remaining patients were diagnosed with either UC (30%) or indeterminate colitis (5%). Eight percent of children diagnosed with CD had perianal fistulas, while 15% had other forms of perianal disease, such as deep fissures, perianal skin tags, and anal distortion. 11342 Fig.3060 describes the disease distribution at diagnosis in this cohort.
Figure 3060 – Disease Distribution in Children With Newly Diagnosed IBD
Adapted from Kugathasan S, Judd RH, Hoffman RG, et al. Epidemiologic and clinical characteristics of children with newly diagnosed inflammatory bowel disease in Wisconsin: A statewide population-based study. J Pediatr. 2003;143(4):525-31.
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Stricture and Perforation in Crohn’s Disease
Most patients with Crohn’s disease (CD) will eventually develop stricture or perforating complications.2000 The distribution of disease location, severity, and complications associated with CD vary widely. As a result, there is a need to classify a patient’s disease to facilitate individualized management.
Cosnes and colleagues2000 performed a retrospective review of a large number of patients
to assess the long-term evolution of the disease behavior of CD, and
to determine the predictive factors of this evolution. Consecutive
patients with CD (2,002 patients) were seen between
From
Figure 492 – Comparison of Behavioral Subgroups of Crohn’s Disease When Defined Without Specific Limit in the Course of the Disease
Table 1 Page 246, Inflamm Bowel Dis 2002;8:(2):244-250. Copyright © reprinted with permission of Wiley-Liss, Inc., a subsidiary of John Wiley & Sons, Inc.
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Figure 493 – Kaplan-Meier Estimates of Remaining Free of Penetrating Complication (Upper Curve) and Free of Stricturing and/or Penetrating Complication (Lower Curve) in 2,002 Patients With Crohn’s Disease Since Onset (Diagnosis) of the Disease. The Number of Patients at Risk Referred to the Number of Patients at Risk for Any Complication.
Figure 1 Page 247, Inflamm Bowel Dis. 2002;8(2)244-250. Copyright © reprinted with permission of Wiley-Liss, Inc., a subsidiary of John Wiley & Sons, Inc.
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Cosnes, et al., demonstrated that the disease behavior of CD is dictated by the duration and location of the disease. Penetrating disease is by far the most frequent ultimate expression of CD, regardless of its location. Stricturing disease is less common. Inflammation should not be considered a specific phenotype and is not a protracted condition.2000
Some studies have suggested that the behavior of the disease does not change with time. Greenstein, et al., for example, found that patients who underwent surgery for perforating complications were vulnerable for further perforation, whereas patients who underwent surgery for stricture disease were at risk for additional stricture formation.2001 The implication of these data is that the transmural inflammatory process of CD will lead, in time, to the unavoidable formation of stenosis and/or fistulas.
Fistulas in Crohn’s Disease
Due to the transmural nature of the inflammation, sometimes ulcers caused by Crohn’s disease (CD) will channel through the gut wall to adjacent areas, including the skin, bladder, vagina, intestine, and perianal areas. These occurrences are known as fistulas and may require medical or surgical therapy.
Fistulas are a common complication of CD, occurring in 30% to 50% of patients over time.9739
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Enteroenteric (bowel-to-bowel) fistulas may be asymptomatic or present as a palpable mass, bacterial overgrowth, or voluminous diarrhea. A significant amount of absorptive surface can be bypassed with an enteroenteric fistula, resulting in weight loss.
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Recurrent urinary tract infections, especially with multiple organisms, or a complaint of pneumaturia suggest a bowel-to-bladder (enterovesical) fistula.
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A psoas muscle abscess and/or renal involvement with ureteral obstruction and hydronephrosis can result from retroperitoneal fistulas.
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Passage of gas or feces through the vagina can be caused by enterovaginal fistulas.
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Bowel contents draining to the surface of the skin are symptomatic of enterocutaneous fistulas.
A population-based study was undertaken in Olmsted County, Minnesota in an attempt to gain knowledge about the cumulative incidence and natural history of fistulas in CD. This unique opportunity was possible, as virtually all of the county residents were treated in 1 of 2 health care organizations (Mayo Clinic and its affiliates and Olmsted Medical Center), both of which have linked records. From this medical records review, 175 patients were diagnosed with CD between 1970 and 1993 and 169 granted permission for review of their records. The data gathered over this 20-year period provided information on occurrence and prevalence. The cumulative risk of 1 fistula (any site) at 1 year was 21.0% (CI 14%-27%). This percentage increased over time and at 5 years was 26% (CI 19%-32%), at 10 years was 33% (CI 25%-40%), and at 20 years was 50% (CI 35%-51%).2002
The location of the fistulas in this assessment are noted in Fig.495.
Figure 495 – Percentage of Fistulae by Type
Figure 3. Page 877, Gastroenterology. 2002;122(4) is used with permission of Elsevier Inc. All rights reserved.
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In a separate study, depicted in the graph Fig.496, Bell, et al., described a total of 169 fistulas in a cohort of 87 patients.
Figure 496 – Anatomical Distribution of Fistulae in Patients With Crohn’s Disease
Adapted from Bell SJ, Williams AB, Wiesel P, et al. The clinical course of fistulating Crohn’s disease. Aliment Pharmacol Ther 2003;17:1145-1151.
Some figures may not display clearly when rendered as a PDF or printed.
Content on this page was last reviewed on October 31, 2009.
Content on this page was last changed on October 12, 2009.
References:| 2000. | Cosnes J, Cattan S, Blain A, et al. Long-term evolution of disease behavior of Crohn’s disease. Inflamm Bowel Dis. 2002;8(4):244-250. |
| 2001. | Greenstein AJ, Lachman P, Sachar DB, et al. Perforating and non-perforating indications for repeated operations in Crohn’s disease: evidence for two clinical forms. Gut . 1988;29(5):588-592 . |
| 2002. | Schwartz DA, Loftus EV, Tremaine WJ, et al. The natural history of fistulizing Crohn’s disease in Olmsted County, Minnesota. Gastroenterology. 2002;122(4):875-880. |
| 2034. | Sands BE. From symptom to diagnosis: clinical distinctions among various forms of intestinal inflammation. Gastroenterology. 2004;126(6):1518-1532. |
| 2112. | Legnani P, Kornbluth A. Clinical features, course and laboratory findings in ulcerative colitis. In: Lichtenstein GR. The Clinician’s Guide to Inflammatory Bowel Disease. Thorofare, NJ: Slack Inc; 2003:27-39. |
| 9739. | Bell SJ, Williams AB, Wiesel P, Wilkinson K, Cohen RC, Kamm MA. The clinical course of fistulating Crohn’s disease. Aliment Pharmacol Ther. 2003;17(9):1145-1151. |
| 11342. | Kugathasan S, Judd RH, Hoffman RG, Wisconsin Pediatric Inflammatory Bowel Disease Alliance. Epidemiologic and clinical characteristics of children with newly diagnosed inflammatory bowel disease in Wisconsin: A statewide population-based study. J Pediatr . 2003;143(4):525-31. |
| 11432. | Baldassano RN, Piccoli DA. Inflammatory bowel disease in pediatric and adolescent patients. Gastroenterol Clin North Am . 1999;28(2):445-458. |
| 11433. | Gupta N, Bostrom AG, Kirschner BS, et al. Presentation and disease course in early- compared to later-onset pediatric Crohn’s disease. Am J Gastroenterol . 2008;103(8):2092-2098. |
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